We describe the results of a systematic review of the literature on occupational therapy–related interventions for people with amyotrophic lateral sclerosis (ALS). The review included 14 studies. We found limited to moderate evidence that people involved in multidisciplinary programs have longer survival than those in general care and limited evidence that those in multidisciplinary programs have a higher percentage of appropriate assistive devices and higher quality of life in social functioning and mental health. Limited evidence indicates that people with ALS are satisfied with the comfort and ease of use of their power wheelchairs (PWCs). In addition, limited evidence is available that PWCs allow people to have increased interaction in the community. Evidence also is limited that some assistive devices are more helpful than others. Moderate evidence indicates that a home exercise program of daily stretching and resistance exercise results in improved function. The implications for practice, education, and research are discussed.